Pediatric Small Bowel Transplantation: An Update
Most pediatric patients with irreversible intestinal failure are dependent on parenteral nutrition (PN) and eventually develop life-threatening complications from long-term PN. Intestinal transplantation is now an accepted treatment for the pediatric popu
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Aparna Rege and D. Sudan
Abbreviations ACR AMR CMV CR EBV GVHD PN PTLD rTAG SBTx
Acute cellular rejection Antibody-mediated rejection Cytomegalovirus Chronic rejection Epstein-Barr virus Graft versus host disease Parenteral nutrition Posttransplant lymphoproliferative disease Rabbit antithymocyte globulin Small bowel transplant
Background Since its conception in the 1960s, there has been considerable growth in the utilization of intestinal transplantation for the management of patients with intestinal failure. Intestinal failure results from the inability of the gut to absorb fluids or nutrients either due to malfunction or from the lack of adequate absorptive surface secondary
A. Rege, MD (*) • D. Sudan, MD Division of Abdominal Transplant, Department of Surgery, Duke University Medical Center, Durham, NC, USA e-mail: [email protected]; [email protected]
to extensive bowel resection. The most common etiology of intestinal failure in the pediatric population includes conditions collectively labeled as short gut syndrome due to necrotizing enterocolitis (NEC), gastroschisis, volvulus, and intestinal atresia. The remaining etiologies of intestinal failure include functional bowel problems with preserved bowel length comprising a large group of conditions such as Hirschsprung’s disease, neuronal intestinal dysplasia, neuropathic or myopathic pseudo-obstruction, protein-losing enteropathies, microvillous inclusion disease, and other or unspecified conditions. In a 2010 survey describing the population of pediatric patients on the intestinal transplant wait list, 51 % were less than 1 year old at initial listing and 31 % were 1–5 years of age [1]. The most common conditions at listing were gastroschisis (23 %), other SGS (21 %), necrotizing enterocolitis (19 %), volvulus (12 %), and functional bowel syndrome (12 %) [1]. In the presence of a comprehensive multidisciplinary rehabilitation program, majority of patients with short gut are successfully managed with a combination of enteral and parenteral nutrition (PN) and surgical reconstruction wherever indicated. There are few isolated case series reports of long-term survival on PN alone in patients with irreversible intestinal failure [2, 3]. Most patients with irreversible intestinal failure develop life-threatening complications from long-term PN and eventually progress to intestinal transplantation.
© Springer International Publishing Switzerland 2016 R.J. Rintala et al. (eds.), Current Concepts of Intestinal Failure, DOI 10.1007/978-3-319-42551-1_11
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Concept of Intestinal Transplantation The US Centers for Medicare and Medicaid Services, in October 2000, established intestinal, combined liver/intestinal, and multivisceral transplantation as the standard of care for patients with intestinal failure when total parenteral nutrition (TPN) therapy has failed [4]. According to Medicare, criteria for TPN failure include impending or overt liver failure from TPNinduced cholestasis, loss of venous access due to thrombosis of m
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